Myasthenia gravis in monozygotic twins. Clinical follow-up nine years after thymectomy
N. Allen, P. Kissel, D. Pietrasiuk and M. J. Perlow
Genetically proved monozygotic female twins in whom myasthenia gravis
developed in their 20s initially had their disease well controlled with
anticholinesterase medication. Because of increasing resistance to
medication, twin 1 had her thymus removed, after which the symptoms
decreased. Predicated on the improvement in her sister and the need for
increasing medication to allay symptoms, and after proof of monozygosity,
twin 2 also underwent thymectomy, with subsequent symptomatic improvement.
The twins were followed up nine and six years after thymectomy,
respectively. Monozygotic twins with myasthenia gravis are generally young
women, with onset of disease in one occurring within one to three years of
the other. Reports of only one affected twin may be misleading because of
inadequate documentation of monozygosity, absence of long-term observation,
or both. Serial investigations of the "uninvolved" twin in a monozygous
pair and proof of monozygosity should be obtained to aid in early diagnosis
and treatment of this illness, as well as to study the pathogenesis of
myasthenia gravis prior to symptom onset.